Leprosy in Six Isolated Residentsof Northern LouisianaTime-Clustered Cases in an Essentially Nonendemic AreaBurton C. West, MD; John R. Todd, MD; Cynthia H. Lary;Leslie A. Blake; Marjorie E. R. Fowler, MD; John W. King, MD

\s=b\Northern Louisiana has been essentially free of indige-nous leprosy, and now it is not. Six new cases of leprosy havebeen diagnosed: three in 1986, the other three in 1985, 1983,and 1982, respectively. The patients had been lifelong resi-dents of six scattered rural parishes. Leprosy had never beenreported from five of them. No patient had had contact withhuman leprosy. The patients were white; four were women;the mean\m=+-\SDage at onset was 60.3\m=+-\16.4years (age range,31 to 80 years); and the mean\m=+-\SDinterval to diagnosis was

1.2 \m=+-\1.4years. One patient had Hodgkin's disease at the ageof 25 years and leprosy at the age of 31 years; another patienthad cervical carcinoma. All rural northern Louisiana residentscoexist with armadillos (Dasypus novemcinctus), some ofwhich are infected with Mycobacterium leprae, the signifi-cance of which is unknown. Hypothetically, exposure to an

unknown human case, reactivation of "asymptomatic" leprosythrough immunosenescence or immunosuppression, or infec-tion from an environmental source might have occurred.Because the patients lacked contact, travel, residence, andexposure risk factors, the origin of leprosy in the new indige-nous cases is noteworthy and is not understood.

(Arch Intern Med 1988;148:1987-1992)

Teprosy is rare in persons born in the United States and ^ is virtually nonexistent in those who have never livedin an area endemic for leprosy.1·2 Twenty to 37 cases ofendemic leprosy are reported per year in the United States.They account for about 10% of the newly reported cases.Endemic leprosy occurs in persons living in Hawaii, Cali¬fornia, Texas, and southern Louisiana.2·3 Northern Louisi-

Accepted for publication April 28, 1988.From the Section of Infectious Diseases, Department of Medicine (Drs

West, Todd, and King and Mss Lary and Blake) and Department ofPathology(Dr Fowler), Louisiana State University School of Medicine, Shreveport.Ms Lary is now with the Division of Infectious Diseases and Immunology,Department of Pediatrics, University of Arkansas for Medical Sciences,Little Rock.

This study was previously published as an abstract in the Program andAbstracts of the 27th Interscience Conference on Antimicrobial Agents andChemotherapy, New York, Oct 4-7, 1987.

Reprint requests to Section of Infectious Diseases, Department ofMedicine, Louisiana State University School of Medicine, PO Box 33932,Shreveport, LA 71130-3932 (Dr West).

ana is not known as a place where transmission of leprosyoccurs or for indigenous cases of leprosy.4·5 It is generallythought that transmission of leprosy requires contact witha case of human leprosy or travel or residence in a regionendemic for leprosy, which is presumed to allow transmis¬sion from unrecognized cases. However, 50% to 70% ofcases of leprosy do not have an identifiable contact with ahuman being with leprosy, making other explanationsplausible.1·6 Exposure to armadillos, which are common inthe south central region of the United States, has recentlybeen shown to be greater in patients with lepromatousleprosy than in controls.7 We report six recent cases ofleprosy in lifelong or nearly lifelong residents of northernLouisiana.

PATIENTS AND METHODSWe investigated six patients recently diagnosed by us or who

were referred to us with leprosy. We performed a complete medicalhistory and physical examination; reviewed medical records;obtained and reviewed laboratory tests, x-ray films, and biopsyspecimens; conducted visits to each patient's home, sometimes onseveral occasions; and interviewed patients and family members,particularly about risk factors for leprosy.

We determined the number of cases of leprosy from each parishor county by a review of state health department records forLouisiana, Mississippi, and Texas and of available records fromthe Gillis W Long Hansen's Disease Center at Carville, La, byconversations with patients, physicians, and others, and by aliterature review.

REPORT OF CASESClinical and Demographic Features

The widely scattered residences of the six patients with leprosyare shown on the map of northern Louisiana (Figure). The clinicaland demographic features of the patients with leprosy are pre¬sented in Table 1. Risk factors for leprosy as related to each ofthe six cases are summarized in Table 2. Additional clinical detailsand risk factor analysis are provided for each case in the "Appen¬dix" section.

Common CharacteristicsEach of the six patients with leprosy was actually or virtually

a lifelong resident of the same immediate area in six parishes innorthern Louisiana. Two patients never moved from their birth-

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place. Five parishes had never before reported leprosy in anyresident. The cases were separated by at least 30 km (Figure). Allof the patients were married or widowed, and white. Four werewomen. The mean ± SD age at clinical onset was 60.3 ± 16.4 years(age range, 31 to 80 years); the mean ± SD interval to diagnosiswas 1.2 ±1.4 years; and the mean ± SD age at diagnosis was61.5 ±17.1 years. In the analysis of risk factors for leprosy (Table2), the patients lacked contact with any known case of humanleprosy, and travel or residence in an endemic region, one of whichis usually considered a prerequisite for acquiring leprosy. None ofthe patients was at risk for, or had antibodies to, human immu¬nodeficiency virus (HIV). All six patients had had indirect contact,and four admitted to direct contact with armadillos, which havebeen a listed risk factor on the reporting form for leprosy providedby the Centers for Disease Control, Atlanta, since 1976. Arma¬dillos have been found throughout the region, rooting or burrowingin yards and fields or dead and decomposing at the roadside.Therefore, the direct and indirect armadillo contact that we reportwas not unique to the patients, but was rather a common andinevitable experience for most rural residents in this region.

COMMENTAs we saw these six new cases of leprosy from this

essentially nonendemic part of Louisiana during a shortperiod, we determined that while they were separatedfrom each other, they appeared to constitute a regional,time-related cluster of new cases. Because Mycobacteriumleprae has never been cultivated in vitro, innumerableunanswered questions exist about leprosy. The clinical andepidemiologie cluster of leprosy cases that we describe isconsistent with hypotheses that emphasize different as¬

pects of transmission and pathogenesis. Therefore, we

explored several, nonexclusive explanations for their re¬cent diagnosis.

AgingIn some populations where leprosy is decreasing, new

cases occur in a more and more aged population. New casesin children and younger adults decrease disproportionatelyto new cases in aged persons, as leprosy decreases and thearea loses its highly endemic character.1·9·10 That leprosy isa complication of aging is in keeping with our observationthat the mean ± SD age at diagnosis was 61.5 ±17.1 yearsin our cases. However, it is not logical to assert that ourcases mean that leprosy is decreasing in this population,because the cluster of isolated cases that we report repre¬sents an absolute increase in regional incidence from zeroin lifelong residents. Thus, this cluster of leprosy is notconsistent with a decreasing incidence.

Immunosenescence, the immunosuppression that accom¬

panies aging, could make leprosy a complication of aging,and might pertain to five of the cases. For this to haveoccurred, one must postulate the existence of asympto¬matic leprosy or alternatively an increased susceptibilitylate in life following exposure to a human case or anenvironmental source of leprosy.

Case ContactThe traditional concept of leprosy transmission is that it

occurs only after close, prolonged human contact. Thecontact is thought to involve nasal secretions. Skin contactscannot be ruled out. In lepromatous leprosy, nasal mucuscontains millions ofbacilli that are aerosolized. It is thoughtthat when inhaled, these acid-fast bacilli lead to infectionin susceptible persons.6 Such a hypothetical explanationfor our cases requires that exposure to an unknown humancase occurred and that our patients, constituting suscep¬tible persons, contracted leprosy. However, detailed inter¬views, concerning family members, contacts, former own¬ers of the family home, obscure illnesses in household orother contacts, and travel, were elicited from each patientand family. Clearly, no known leprosy case contact existedfor any of them. The possibility of six unknown contacts,ie, six living human beings with untreated active, probablylepromatous, leprosy, each of whom was in regular closeproximity to one of the six patients, appears to have beenhighly unlikely.

Map of northern Louisiana showing residence location of each ofsix sporadic cases of leprosy. MISS indicates Mississippi.

Table 1.—Clinical Features of Six Cases With Leprosy in Northern Louisiana*

Case/Sex

LeprosyOnset Diagnosis

Age, y Year Age, y YearClinical

PresentationLeprosyTypef

UnderlyingCondition

LouisianaParish

1/F2/F

3/F

4/M5/M

6/F

6754

31

6579

66

19801985

1985

19861986

1979

7054

31

6580

69

19831985

1986

19861986

1982

Red macules

Edema, fever

Red papules

Red maculeRed macules

Arthritis

BL

BL

BTLL

LL

NoneCervical

carcinomaHodgkinfe

diseaseNoneNone

Diabetesmellitus

LincolnRural

CaddoCatahoula

SabineWest

CarrollMorehouse

*See "Appendix" section for details.tLeprosy type refers to the Ridley-Jopling8 classification, where LL means lepromatous leprosy; BL, borderline lepromatous leprosy; and BT, borderline

tuberculoid leprosy.

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Table 2.—Risk Factors for Leprosy in Six Cases in Northern Louisiana*

Case

Family Member,Friend, or

AcquaintanceWith Leprosy

Travelout of US

or to EndemicAreas in US

ResidencesIn Addition

to Those Citedin Table 1

LeprosyReported

From HomeParish

Armadillo ContactfDirect Indirect

NoNo

NoNoNoNo

NoNo

No

±*1NoNo

NoneArkansas,

1943-1949, 1951NoneYes**NoneSimpson Court,

Miss before 1956

No

Yes, seeTable 3NoNoNoNo

NoYes, food

Yes, food

Yes, touchYes, touchNo

YesYes

YesYesYesYes

*See "Appendix" section for details. US indicates United States.fFood means that armadillo meat was prepared in the women's kitchens to eat; neither admitted eating it; touch, that armadillos were handled alive or

dead; and indirect, walking barefoot where armadillos rooted and were killed, having armadillos live beneath one's house, drinking water that was potentiallycontaminated with armadillo excrement, or other means.

tCase 4's risk of contact with leprosy cases appeared to be nil.

Similarly, it seems untenable, in attempting to explainthe average age of the cases, that simply later in life thanin a highly endemic area they had had contact with anactive case and contracted leprosy.

Asymptomatic or Subclinical LeprosyThe theory of pathogenesis, favoring the existence of an

asymptomatic or subclinical leprosy, is based on limiteddata.11 The most generalized hypothesis is that nearlyeveryone gets and carries a subclinical leprosy infection.12The idea of asymptomatic leprosy is consistent with wide¬spread but asymptomatic histoplasmosis or tuberculosis inendemic areas, if such a state were to exist, then activationor reactivation of asymptomatic leprosy through immuno-senescence of immunosuppression could occur, and mightexplain isolated cases like these.

If there were an asymptomatic state, immunosenescencecould make leprosy a complication of aging. Asymptomaticleprosy is one way to account for the epidemiologie obser¬vation that in some populations, new cases are observedmostly in older persons.1·9·10 The immune systems of elderlypersons might permit expression of leprosy, like reactiva¬tion tuberculosis that now has its highest incidence in agedpersons. The mean±SD age at diagnosis of 61.5± 17.1years found in our cases is consistent with this possibility.The idea that leprosy is a complication of aging is inkeeping with our observations.

Distinguishing prolonged incubation from asymptomaticleprosy is not easy. Recently, the M leprae-speciftc phenolicglycolipid-1 (PGL-1) antigen was detected in blood from acontact of a lepromatous leprosy case two years before thecontact developed clinical leprosy.

Immunosuppression and Host Factors

Similarly, if there were an asymptomatic state, immu¬nosuppression could cause leprosy to become clinicallyapparent. Asymptomatic leprosy or not, immunosuppres¬sion might increase susceptibility in persons who are

exposed to a human case or to an environmental source,causing them to become ill with leprosy either earlier orin greater numbers than immunologically normal persons.In our patients, an exception to leprosy in aged personswas patient 3 ("Appendix" section); at the age of 31 years,she had already been treated for Hodgkin's disease for 5.5years. Her case was like two other cases of leprosy, whichcomplicated lymphoma four to five years later.13 Further¬more, the simultaneous presentation in our case 2 ("Ap-

pendix" section) of advanced cervical carcinoma and lep¬rosy is remarkable, suggesting a relationship between thetwo; however, an association between leprosy and solidtumors has not been made. The potential for persons withHIV infection or with acquired immunodeficiency syn¬drome to develop opportunistic leprosy should be carefullywatched, but only one case has been reported so far.14 Noneof our patients was at risk for or had antibodies to HIVGenerally, leprosy does not clinically complicate the usualstates of immune compromise, but perhaps this is a

misconception, since until recently few severely compro¬mised persons lived long enough to develop leprosy. Lep¬rosy could, therefore, be a complication of immunosup¬pression, with aging and treated lymphoma being forms ofimmunosuppression that are supported by our observa¬tions.

More broadly, susceptibility to leprosy includes all hostfactors, from nutrition to genes, even if all are mediatedthrough immune systems. Because nonrelatives in a house¬hold with an active lepromatous case get leprosy at a rateof not greater than 5%, genes influence susceptibility.16Contrary to earlier reports,16 HLA antigens, specificallyHLA-DR2 and HLA-DQwl, have been associated withleprosy, an observation that has been extended by meta-analysis to many populations (J.R.T., B.C.W, and J. C.McDonald, MD, unpublished data, 1988).17·18

Environmental Nonhuman SourcesThese patients stimulated our critical review of nonhu¬

man environmental sources for M leprae.6 Infection fromthe environment might have occurred, for such sources,whether primary or intermediate, are plausible in explain¬ing the origin of M leprae for some human leprosy. It isestablished that M leprae exists in the south central UnitedStates in the nine-banded armadillo (Dasypus novemcinc-tus). Since leprosy was induced experimentally in thismammal in 1971,19 and especially since wild armadilloswere discovered to harbor it in 1975,zo there has beenconcern that the armadillo could spread leprosy to humanbeings. Homology has been shown between DNA fromM leprae from a human case and mycobacteria from a

leprous armadillo, thus removing the distinction betweenthe organisms.21 The disease in the two species has simi¬larities.22

Proof that M leprae is spread to human beings by infectedwild armadillos is lacking,6 but there are some worrisomeobservations. In Mexicans with lepromatous leprosy, ex-

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posure to armadillos was significantly greater than incontrols.7 Seven cases of leprosy in men who handledarmadillos in parts of Texas that were known to haveleprous armadillos have been reported, with the strongimplication that leprosy was acquired in some unspecifiedmanner from the armadillos that they handled and wres¬tled.2326

An armadillo with a leprosylike disease was found nearthe home of patient 1; this home was located in theimmediate area where all 27 previously reported northernLouisiana armadillos were trapped for survey (see "Appen¬dix" section). The survey showed that eight (29.6%) of 27had leprosy.22·26·27 In another survey, 494 armadillos thatwere found dead at the roadside in Louisiana were studied,including 13 from northern Louisiana. None of the 13armadillos and ten (2%) of the 494 were positive for lep¬rosy.28·29 In other surveys, two (10%) of 20 armadillos fromcoastal Louisiana were leprous,30 and one of 96 from Mexicowas leprous.31 Of Louisiana armadillo serum samples col¬lected from 1961 to 1964, 17 (9.3%) of 182 and, of recentserum samples, about 20% were positive for specific IgMantibody to PGL-1 antigen.32 This demonstrated the exis¬tence of leprosy in armadillos before their inoculation withM leprae in 1968. Although armadillos were known to be inLouisiana before 1920,33 information about their migrationis scant and the time of acquisition of leprosy is unknown.Environmental concepts of leprosy have been advancedwith the discovery of PGL-1 antigen in soil.6 Earthwormsnear patients' homes, examined as a possible armadillo-leprosy link, however, did not contain acid-fast bacilli.34

The two people who were previously described withlymphoma, complicated by leprosy, live in the same generalregion as our cases, including patient 3 ("Appendix" sec¬tion) who had Hodgkin's disease.13 It is noteworthy thatone patient was from the east Texas town of Lufkin andthe other was from Victoria County, Texas, where morethan 7% of armadillos have leprosy.21 Furthermore, VictoriaCounty is the home of five of the seven reported cases ofleprosy in armadillo handlers.

Throughout parts of its range, the armadillo might be asentinel animal for M leprae in the environment. Althoughit would be incidental to this biologic role, the armadillobrings M leprae in contiguity with rural residents in amanner that was totally uncontemplated 15 years ago.36Most people in this region have driven past a dead arma¬

dillo, every infected one of which is estimated to release 12 M leprae.2*

Possible transmission of leprosy from infected armadillosto human beings has been downplayed because of theextensive casual contact with armadillos that many Loui-sianans have and because of the low incidence of leprosy.36However, if everyone in a population has contact witharmadillos, much of it indirect and unnoticed, then com¬paring the contact in persons with and without leprosy isnot meaningful. Yet, apparently that study did just that,ie, interviewed an age- and sex-matched control subjectfor each rare patient with leprosy from endemic southernLouisiana and drew conclusions.36 The far greater rarity ofleprosy, coupled with the fact that it is essentially notendemic, makes that approach even more problematic innorthern Louisiana than in southern Louisiana.

ConclusionWe have reported six cases of leprosy in virtually lifelong

residents of this essentially nonendemic region. The new¬ness of these clinical cases makes history itself the controlfor our observations. Thus, they are a new, endemic clusterof leprosy. Several unresolved theories of pathogenesis and

transmission of leprosy can be supported in nonexclusiveways with the observations presented. The cases are rural,dispersed, aged, or immunosuppressed, and they havedirect or indirect contacts with armadillos. Everyone inthe region coexists with armadillos, some of which areinfected with M leprae. This contact may pertain in a

currently unknown way to these cases.

This study was supported in part by grants from the Ed E. and GladysHurley Foundation, Shreveport, La, and American Leprosy Missions Ine,Elmwood Park, NJ.

We thank James R. Bergeron, MD, Bruce H. Clements, MD, Lee RoyJoiner, MD, Robert E. Lyon, MD, Ted Rosen, MD, Paul R. Winder, MD,and John E. Wolf, Jr, MD, for referrals or assistance; the personnel of thehealth departments of Louisiana, Mississippi, and Texas, and the Gillis WLong Hansen's Disease Center, Carville, La, especially Barbara AnnMaxwell, for assistance; and Tommie Lue Maddox for secretarial assistance.

References

1. Joseph BZ, Yoder LJ, Jacobson RR: Hansen's disease in native-borncitizens of the United States. Public Health Rep 1985;100:666-671.

2. Neill MA, Hightower AW, Broome CV: Leprosy in the United States,1971-1981. J Infect Dis 1985;152:1064-1069.

3. Redd SC, Collin S, Cohen ML: Leprosy in the United States, 1982\x=req-\1985, in Program and Abstracts of the 27th Interscience Conference onAntimicrobial Agents and Chemotherapy, New York, Oct 5,1987. Washing-ton, DC, American Society for Microbiology, 1987, abstract 410, 168.

4. Feldman RA, Sturdivant M: Leprosy in Louisiana, 1855-1970: Anepidemiologie study of long-term trends. Am J Epidemiol 1975;102:303\x=req-\310.

5. Badger LF: Epidemiology, in Cochrane RG, Davey TF (eds): Leprosyin Theory and Practice. Baltimore, Williams & Wilkins, 1964, pp 69-97.

6. Blake LA, West BC, Lary CH, et al: Environmental non-humansources of leprosy. Rev Infect Dis 1987;9:562-577.

7. Thomas DA, Mines JS, Thomas DC, et al: Armadillo exposure amongMexican-born patients with lepromatous leprosy. J Infect Dis 1987;156:990\x=req-\992.

8. Ridley DS, Jopling WH: Classification of leprosy according to immu-nity: A five-group system. Int J Lepr Other Mycobact Dis 1966;34:255-273.

9. Worth RM, Bomgaars MR: Immigration and leprosy in Hawaii, 1960\x=req-\1981. Int J Lepr Other Mycobact Dis 1982;50:335-341.

10. Irgens LM: Secular trends in leprosy: Increase in age at onsetassociated with declining rates and long incubation periods. Int J LeprOther Mycobact Dis 1985;53:610-617.

11. Taylor CE, Elliston EP, Gideon H: Asymptomatic infections inleprosy. Int J Lepr Other Mycobact Dis 1965;33:716-731.

12. Reich CV: Leprosy: Cause, transmission, and a new theory ofpathogenesis. Rev Infect Dis 1987;9:590-594.

13. Levy ML, Rosen T, Tschen JA, et al: Hansen's disease followinglymphoma. J Am Acad Dermatol 1986;15:204-208.

14. Lamfers EJP, Bastiaans AH, Mravunac M, et al: Leprosy in theacquired immunodeficiency syndrome. Ann Intern Med 1987;107:111-112.

15. Binford CH, Meyers WM, Walsh GP: Leprosy. JAMA 1982;247:2283\x=req-\2292.

16. Tiwari JL, Terasaki PI: HLA and Disease Associations. New York,Springer-Verlag NY Ine, 1985, pp 383-401.

17. Sehauf V Ryan S, Scollarci D, et al: Leprosy associated with HLA\x=req-\DR2 and DQwl in the population of northern Thailand. Tissue Antigens1985;26:243-247.

18. Kim SJ, Choi IH, Dahlberg S, et al: HLA and leprosy in Koreans.Tissue Antigens 1987;29:146-153.

19. Kirchheimer WF, Storrs EE: Attempts to establish the armadillo(Dasypus novemcinctus Linn) as a model for the study of leprosy: I. Reportof lepromatoid leprosy in an experimentally infected armadillo. Int J LeprOther Mycobact Dis 1971;39:693-702.

20. Walsh GP, Storrs EE, Burchfield HP, et al: Leprosy-like diseaseoccurring naturally in armadillos. J Reticuloendothel Soc 1975;18:347-351.

21. Smith JH, Folse DS, Long EG, et al: Leprosy in wild armadillos(Dasypus novemcinctus) of the Texas gulf coast: Epidemiology and myco-bacteriology. J Reticuloendothel Soc 1983;34:75-88.

22. Binford CH, Meyers WM, Walsh GP, et al: Naturally acquired leprosy\x=req-\like disease in the nine-banded armadillo (Dasypus novemcinctus): Histo-pathologic and microbiologie studies of tissues. J Reticuloendothel Soc1977;22:377-388.

23. Freiberger CF, Fudenberg HH: An appetite for armadillo. HospPract 1981;16:137, 141, 144.

24. Lumpkin LR III, Cox GF, Wolf JE Jr: Leprosy in five armadillohandlers. J Am Acad Dermatol 1983;9:899-903.

25. Lumpkin LR III, Cox GF, WolfJE Jr: Leprosy in armadillo handlers.J Am Acad Dermatol 1984;10:1073.

26. Walsh GP, Storrs EE, Meyers W, et al: Naturally acquired leprosy\x=req-\

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like disease in the nine-banded armadillo (Dasypus novemcictus): Recentepizootiologic findings. J Reticuloendothel Soc 1977;22:363-367.

27. Meyers WM, Walsh GP, Binford CH, et al: Indigenous leprosy innine-banded armadillos, in Thompson ES (ed): The Armadillo as an

Experimental Model in Biomedical Research, scientific publication 366.Washington, DC, Pan American Health Organization, 1978, pp 67-76.

28. Job CK, Harris EB, Allen JL, et al: A random survey of leprosy inwild nine-banded armadillos in Louisiana. Int J Lepr Other Mycobact Dis1986;54:453-457.

29. Job CK, Harris EB, Allen JL, et al: Thorns in armadillo ears andnoses and their roles in the transmission of leprosy. Arch Pathol Lab Med1986;110:1025-1028.

30. Smith JH, File SK, Nagy BA, et al: Leprosy-like disease of wildarmadillos in French Acadiana, Louisiana. J Reticuloendothel Soc1978;24:705-719.

31. Amezcua ME, Escobar-Guti\l=e'\rrez A, Storrs EE, et al: Wild Mexicanarmadillo with leprosy-like infection. Int J Lepr Other Mycobact Dis1984;52:254-255.

32. Truman RW, Shannon EJ, Hagstad HV, et al: Evaluation of the originof Mycobacterium leprae infections in the wild armadillo Dasypus novem-cinctus. Am J Trop Med Hyg 1986;35:588-593.

33. StreckerJK: The extension of the range of the nine-banded armadillo.J Mammalogy 1926;7:206-225.

34. Blake LA, West BC, Lary CH, et al: Earthworms near leprosypatients' homes are negative for acid-fast bacilli by Fite stain, providing nolink between leprous armadillos (Dasypus novemcinctus) and humanleprosy. Microbial Ecol, in press.

35. Walsh GP, Meyers WM, Binford CH: Naturally acquired leprosy inthe nine-banded armadillo: A decade of experience, 1975-1985. J LeukocyteBiol 1986;40:645-656.

36. Filice GA, Greenberg RN, Fraser DW: Lack of observed associationbetween armadillo contact and leprosy in humans. Am J Trop Med Hyg1977;26:137-139.

APPENDIX: CLINICAL SUMMARY AND ANALYSIS OF RISKFACTORS FOR SIX CASES OF LEPROSY

Case 1.—This patient presented for a red macule on her thighthat was noted more than one year earlier. It enlarged, a new onebecame visible, and both were anesthetic. A skin biopsy specimenshowed chronic granulomatous inflammation with numerous acid-fast bacilli, consistent with borderline lepromatous leprosy. FromLouisiana State University Hospital, Shreveport, she was referredto the Gillis W Long Hansen's Disease Center in Carville, La. Shereceived outpatient treatment for three years with dapsone andrifampin, and she has continued treatment with dapsone. She hasnearly recovered fully.

This patient had had no risk factor for leprosy, except contactwith armadillos. She was born on a farm and had lived there herentire life. Armadillos were ubiquitous there after 1940, and wereregarded as a nuisance. They burrowed by the shallow well andunder her house, which had floorboards between which dust couldenter. Her husband shot many armadillos in their yard, spillingblood and discarding bodies nearby. She often went barefoot wherethe armadillos rooted and were killed. Near the house was a worm"farm," ie, soil kept moist to grow fishing worms, which attractedarmadillos. That dirt got trafficked inside. Hence, various humancontacts with armadillos, their blood, and other body fluidsoccurred.

In December 1985, biopsy specimens were taken from anarmadillo that was killed nearby by a vehicle but not mutilated.Ear specimens that were fixed, sectioned, and stained (hema¬toxylin-eosin) showed no granulomas or inflammation, but a Fitestain revealed numerous acid-fast bacilli singly and in clusters,typical of armadillo leprosy. Of note is that in the survey ofnorthern Louisiana armadillos, all were trapped in this area, andeight (29.6%) of 27 had leprosy.

Case 2.—This patient was referred to the Louisiana StateUniversity Hospital for cervical carcinoma. In consultation forfever, we found peculiar ankle edema and chronic erythema. Askin biopsy specimen showed foamy macrophages; a Fite stainshowed numerous acid-fast bacilli, some within cutaneous nerves.Many erythematous lesions and extensive edema of both legsquickly developed. She was treated with dapsone and rifampinwhile undergoing cancer therapy. After developing jaundice anderythema nodosum leprosum, she was referred to the Gillis WLong Hansen's Disease Center, where rifampin was discontinued.Skin scrapings showed numerous acid-fast bacilli, consistent withlepromatous leprosy. She has had a gradual response to dapsone.

Risk factors included her birth in Caddo Parish, Louisiana, theonly parish in this report from which leprosy has previously beenreported, and armadillo contact. Caddo Parish is urban becauseof Shreveport; however, she was born and lives in a rural area,50 km from Shreveport. She lived for six years in Arkansas, astate with no endemic leprosy. Ofeight cases reported from CaddoParish, only one other was from a rural area like case 2 (Table 3);that Texan lived in Caddo Parish after the age of 2 years, exceptfor a year in Wichita Falls, Tex, and several months in San Antonio,Tex (1918), at which time he was healthy, but where he might havebeen exposed. Lepromatous leprosy was found in 1919, andreported in 1924 from a town that no longer exists. He had had noknown leprosy contacts. He had leprosy from 1919 to 1924, whileliving 16 km from where our patient 2 was born in 1931. Thispatient, her parents, and friends who were born there in 1899could not establish any contact or connection with him, usingnames and places. No other case occurred there. Other cases wereimported or appeared to be irrelevant to case 2 and each other.Hence, evidence does not favor human-to-human transmission ofleprosy in Caddo Parish.

This patient first saw armadillos as a child. They burrowedunder her home and were sometimes destroyed in the yard. Theywere active as shown by burrows and rooting signs, attracted byworms and a bayou. The spring was surrounded by armadilloburrows. She often went barefoot. A year before presentation, shelacerated her ankle on a stump. The unhealed ankle was scratchedby a rooster while she was barefoot in the chicken yard. Armadillosfrequented these areas. The wound had not healed when shepresented; it was the first site affected by clinical leprosy, and ithealed with antileprosy drug treatment.

Case 3.—This patient observed red papules on her ankle. In amonth, edema developed, and the papules became tender, causingher to present to Louisiana State University Hospital in November1985. She thought they might be Hodgkin's disease, which hadbeen diagnosed in 1980. She had undergone a staging laparotomy

Table 3.—Analysis of Case 2 With Reference to Leprosy in Caddo Parish, Louisiana*

Case/Sex Birthplace, ytPlace Reported

From, yYear of Deathor Follow-up^ Comment

1/M2/M3/F4/M5/F6/F7/F8/F

Lufkin, Tex, 1907Caddo ParishCaddo ParishCaddo ParishIndia, 1942

Louisiana, 1934

Burma, 1933Our case 2, 1931

Caddo City, La, 1924Keithville, La, 1925Shreveport, La, 1932

Shreveport, 1954

Shreveport, 1959Shreveport, 1982Shreveport, 1984

Vivian, La, 1985

Died, 1935Died, 1926Died, 1933Follow-up, 1959, NEAL

Follow-up, 1979, NEAL

Follow-up, 1985, NEAL

Follow-up, 1984, NEAL

Follow-up, 1988

See text; long visit to San Antonio, Tex, in 1918Shreveport area, 55 km from our case 2

Manila, Philippines; Korea; Hawaii in World War IIFather had leprosyLived >2 y in PhilippinesCame to US in 1971

Dapsone treatment; see text

*Data from the Gillis W. Long Hansens Disease Center, Carville, La, referring physicians, and local medical records.fBirthdate unknown for patients 2 through 4.INEAL indicates no evidence of active leprosy.

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with splenectomy, responded to chemotherapy, and relapsed in1982. A second course of chemotherapy caused remission.

Skin biopsy specimens showed granulomatous inflammation andacid-fast bacilli (Fite stain) in extracellular clusters, in macro¬phages, and inside cutaneous nerves, consistent with lepromatousleprosy. The papules became larger, more numerous, and moretender, and they appeared on her arms and face. Therapy withdapsone and rifampin has led to resolution of the skin lesions.

Never before has leprosy been reported from Catahoula Parish,Louisiana, but a man born there in 1934 was reported to be fromSt Landry Parish, Louisiana, in 1982. This white man was born25 km from the home of patient 3, but from 1946 to 1958, he livedin Rapides Parish, Louisiana, and from 1958 in St Landry Parish.Those parishes are known for endemic leprosy and are consideredthe source of his leprosy. Risk factors appeared to be limited toarmadillos. This patient had lived on one rural property. Armadil¬los lived there in a slough and burrowed near the well. She hadlived partly at the home of her parents-in-law. Armadillos livednear it and under it, partly attracted by a fishing bait worm farm.Her father-in-law had killed a "boxcarful" of armadillos; he dis¬posed of them nearby.

Case 4.—This patient presented to the Louisiana State Univer¬sity Hospital with a red 5-cm papular lesion on his left shoulder,which had begun three months earlier as an erythematous ringlikemacule. He had had the onset of a 10-kg weight loss five monthsearlier, and swelling of his left hand and hyperuricemia one monthbefore admission, which was treated elsewhere as gout. He alsohad small red papules on his left arm, trunk, and abdomen;erythematous macules numbered ten each on his left arm andchest and six on his right arm. A severe, mainly motor neuropathyin his left arm resulted in loss of muscle mass and was particularlynoticeable because he was a carpenter and guitarist. A left brachialarteriogram showed obstruction of the ulnar artery, increasedvascularity around the distal ulna and in the hand, and advancedbone erosions in the hand, consistent with inflammation, synovitis,and periarticular vasculitis. Skin biopsy specimens showed gran¬ulomatous inflammation, but only the seventh specimen showedthree beaded acid-fast bacilli (Fite stain), thus confirming border¬line tuberculoid leprosy. To dapsone and rifampin treatment wasadded prednisone for management of neuropathy. His skin lesionsand left arm edema have gradually improved; the neuropathy hasnot progressed.

This patient was born and lived in rural Sabine Parish, Louisi¬ana, from which leprosy has never before been reported. He alsolived in Texas at Anáhuac, Chambers County, from 1952 to 1962.Two men with leprosy were reported from Chambers County. Thefirst had come from Vacherie, La (born in 1911), and lived in Hank-amer in Chambers County when his leprosy was diagnosed in 1935;he was treated at Carville and was unavailable for follow-up in1960; his brother died of leprosy at Carville. The second was a Mex¬ican laborer (born in 1941) whose leprosy was diagnosed in 1976.Both contracted it elsewhere, and the one antedating the patient

(case 4) did not live in Anáhuac. Case 4 also lived at Fort Hood,Bell County, Tex, from 1941 to 1946, which also appeared to beirrelevant because the first case from Bell County was in 1973.

As a carpenter who worked outdoors near his home, he was wellaware of having indirect contact with armadillos in his ruralenvironment. Some lived near his home, burrowing near hisshallow water well.

Case 5.—This patient was an active man who had onset ofsplotchy red macules on his trunk in April 1986. Within twomonths, he had nodular red lesions on his torso and macular andpapular red skin lesions on all extremities. His internist suspectedleprosy. A skin biopsy specimen showed inflammation in thedermis, perineural inflammation, and macrophages with largenumbers of acid-fast bacilli (Fite stain). The diagnosis of leprom¬atous leprosy was classified as subpolar at the Gillis W LongHansen's Disease Center. After five months of dapsone andrifampin treatment, erythema nodosum leprosum developed andlater resolved.

Patient 5 has lived only in West Carroll Parish, Louisiana.Leprosy had never been reported from this rural parish before,although a previously described patient with leprosy from the cityof Monroe, La (Ouachita Parish), in 1980 had been born in WestCarroll Parish, Louisiana, in 1947. That white man served in theUS Navy and went to ports of call in leprosy-endemic countries.Patient 5 first saw an armadillo in 1914. It was alive and displayedas a novelty by local hunters. In both sport and employment thispatient was outdoors, so he had had direct and indirect contactwith armadillos since they became common in the 1930s. Thisincluded killing two to three armadillos weekly near his homesand disposing of the carcasses by hand.

Case 6.—This elderly diabetic patient had had the onset, poorlyremembered, of a discolored lesion on a wrist, for which she saw a

physician in 1979. Arthritis was diagnosed, for which she receivedsteroid and other therapy. From 1979 to 1981, the rash extended.In 1982, a dermatologist found an erythematous maculopapulardermatitis of her face and extremities and diagnosed lepromatousleprosy with skin biopsy specimens. The Gillis W Long Hansen'sDisease Center confirmed it, and therapy with dapsone andrifampin was begun. She discontinued taking rifampin after 18months; many purple nodules developed that suggested Kaposi'ssarcoma to dermatologists, but she had lepromatous leprosy. Poorcompliance contributed to her relapse. Since 1986, she has re¬sponded to the addition of clofazimine to dapsone treatment.

The risk factors in patient 6 were limited. She lived in ruralSimpson County, Mississippi, before moving to a town in More-house Parish, Louisiana, in 1956. Before her, no one with leprosyhas been known to have been born in or reported to be fromSimpson County or Morehouse Parish. She had definite indirectcontact with armadillos while hunting and fishing with herhusband from 1956 until his death in 1974. She had had no contactwith armadillos at her home or yard, having lived in an olderframe house in town for 32 years.

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